Renal disease is a well-recognized manifestation of cystic fibrosis (CF) and people with CF are at increased risk of nephrolithiasis. Lithotripsy is the preferred treatment but has occasionally been associated with pulmonary complications. Here we report the case of a person with CF who developed a pneumothorax soon after lithotripsy and discuss the potential mechanism of injury. We hope this case highlights some of the additional considerations clinicians should take into account when managing patients with advanced pulmonary disease in CF.
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http://dx.doi.org/10.1016/j.chest.2018.03.023 | DOI Listing |
Am J Physiol Lung Cell Mol Physiol
January 2025
Division of Pulmonology, Asthma, Cystic Fibrosis, and Sleep, Emory University School of Medicine, Atlanta, GA, USA.
Secondhand smoke exposure (SHSe) is a public health threat for people with cystic fibrosis (CF) and other lung diseases. Primary smoking reduces CFTR channel function, the causative defect in CF. We reported that SHSe worsens respiratory and nutritional outcomes in CF by disrupting immune responses and metabolic signaling.
View Article and Find Full Text PDFPediatr Pulmonol
January 2025
University Hospitals Cleveland Medical Center, Division of Pulmonary, Critical Care and Sleep Medicine, Cleveland, Ohio, USA.
Objective: Although studies have examined changes in C-reactive protein (CRP) during pulmonary exacerbations (PEX) in people with cystic fibrosis (PwCF), few have evaluated CRP profiles across age groups. Here, we characterize age-related CRP responses to PEX treatment.
Methods: We measured CRP concentrations at the beginning and end of intravenous (IV) antibiotic therapy for PEX in 100 pediatric and 147 adult PwCF at 10 US CF Centers.
Pediatr Pulmonol
January 2025
Centre de Ressources et de Compétences pour la Mucoviscidose, CHU Timone-Enfants, Marseille, France.
Introduction: Cellular characteristics of induced sputum (IS) are not investigated in cystic fibrosis (CF) patients.
Objectives: This pilot study, conducted on 17 expectorating CF adolescents, compared sputa obtained the same day, in a stable period, by autogenic drainage (expectorating sputum, ES) and 4 h later after inhaling hypertonic saline (IS).
Results: No difference was noted concerning weight, volume, and percentage of dead cells between the two collection methods.
Front Immunol
January 2025
Inflammation and Immunity in Global Health Program, Research Institute of the McGill University Health Centre, Montreal, QC, Canada.
Background: Factors leading to severe COVID-19 remain partially known. New biomarkers predicting COVID-19 severity that are also causally involved in disease pathogenesis could improve patient management and contribute to the development of innovative therapies. Autophagy, a cytosolic structure degradation pathway is involved in the maintenance of cellular homeostasis, degradation of intracellular pathogens and generation of energy for immune responses.
View Article and Find Full Text PDFCureus
December 2024
Internal Medicine, Kettering Health Network, Kettering, USA.
Aquagenic syringeal acrokeradermatoma (ASA) is a dermatological condition characterized by the transient appearance of edematous, white, translucent papules on the palms, typically triggered by water exposure. While ASA is most commonly associated with cystic fibrosis (CF) and predominantly affects young females, there has been a significant increase in ASA cases since the most recent update in 2015. The COVID-19 pandemic increased the number of patients diagnosed with ASA following exposure to the viral infection.
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